Talk:Glutathione synthetase deficiency

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removal of ad in external links section[edit]

I removed this ad for very expensive supplements: The Benefits Of Glutathione Riboceine http://www.glutathioneriboceine.com/ in the external links section. Inexpensive selenium and vitamin D3 are the only supplements that might improve the bio-availability of your glutathione. You can search for glutathione and selenium or glutathione and vitamin D3 aka Cholecalciferol on Google or PubMed.

I quote a paragraph in the glutathione entry in wikipedia: Glutathione is only to a small extent bioavailable to humans; the human body is capable of maintaining a consistent level of GSH. Oral introduction of GSH into the body is, in fact, scarcely effective to increase its plasma and/or intracellular concentration. At the base of its poor bioavailability is the nature of glutathione which, being a tripeptide, is the substrate of proteases (peptidases) of the alimentary canal, and the absence of a specific carrier of glutathione at the level of cell membrane.[1][2]

While this earlier article seems to argue for this supplement, it is in mice, and the link is still an ad, and it smells like a paid study. http://onlinelibrary.wiley.com/doi/10.1111/j.1742-7843.2005.pto_96613.x/pdf

References

  1. ^ Allen, J; Bradley, RD (Sep 2011). "Effects of oral glutathione supplementation on systemic oxidative stress biomarkers in human volunteers". J Altern Complement Med. 17 (9): 827–33. doi:10.1089/acm.2010.0716.
  2. ^ Witschi, A; Reddy, S; Stofer, B; Lauterburg, BH (1992). "The systemic availability of oral glutathione". Eur J Clin Pharmacol. 43 (6): 667–9. doi:10.1007/bf02284971. PMID 1362956.

glutathione/cysteine[edit]

Has anyone seen the orphanet webpage for glutathione synthase deficiency? <http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=1852094> It says this : "N-Acetylcysteine used to be recommended because it may protect cells from oxidative stress. However, cysteine has been shown to accumulate in tissues of patients with glutathione synthetase deficiency – at least in cultured fibroblasts [24]. Since cysteine is known to be neurotoxic in excessive amounts [32], treatment with N-acetylcysteine should not be recommended for patients with glutathione synthetase deficiency as this may increase the intracellular cysteine levels even more."

Is this true? This goes against decades of knowledge about this disorder. It says that these people with GSD shouldn't take cysteine b/c it accumulates in their tissues and is neurotoxic. Is this true... I've never heard this before. —Preceding unsigned comment added by Interestedperson (talkcontribs) 15:47, 1 May 2008 (UTC)[reply]

These patients should be taking liposomal glutathione, presumably they could live a normal life if they took it each day, why has no one realized this? Sellingstuff (talk) 06:47, 24 April 2015 (UTC)[reply]